Key result
Intensified dietary restriction safely controls severe hypertriglyceridemia from ApoC-II deficiency during pregnancy.
Why the study?
Evidence guiding management of apolipoprotein C-II deficiency during pregnancy is limited.
Case Report (n=1)
Intensified dietary restriction and appropriately timed delivery can successfully manage severe biphasic hypertriglyceridemia in pregnant women with ApoC-II deficiency without the need for medical therapy or plasma exchange.
Apolipoprotein C-II (ApoC-II) deficiency is a rare genetic cause of familial chylomicronemia syndrome (FCS) that predisposes affected individuals to severe hypertriglyceridemia and acute pancreatitis. Evidence guiding management during pregnancy is limited. We report a woman with ApoC-II deficiency (ApoC-II Wakayama mutation) who showed a biphasic triglyceride (TG) elevation. TG increased markedly in the second trimester, improved with intensified dietary restriction, and rose again to 1400 mg/dL (15.8 mmol/L) in the third trimester. Because she remained asymptomatic and TG had responded to dietary therapy, medical therapy and plasma exchange were not initiated. Labour was induced at 36 weeks, resulting in uncomplicated vaginal delivery and rapid postpartum TG reduction. This case highlights trimester-specific monitoring and individualized reassessment of dietary, medical, plasma exchange, and delivery strategies in FCS.
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Murai et al. (2026) conducted a case report in Apolipoprotein C-II deficiency / familial chylomicronemia syndrome in pregnancy (n=1). Intensified dietary restriction was evaluated on Triglyceride levels and pregnancy outcome. Intensified dietary restriction successfully managed biphasic hypertriglyceridemia (peak 1400 mg/dL) in a pregnant woman with ApoC-II deficiency, leading to an uncomplicated delivery at 36 weeks.
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